J Clin Neurol.  2020 Jul;16(3):461-469. 10.3988/jcn.2020.16.3.461.

Clinical Spectrum of Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G-Associated Disease in Korean Children

Affiliations
  • 1Department of Pediatrics, College of Medicine, The Catholic University of Korea, St. Vincent's Hospital, Suwon, Korea.
  • 2Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
  • 3Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.
  • 4Department of Pediatrics, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Korea.
  • 5Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
  • 6Department of Biochemistry and Molecular Biology, and Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Korea.

Abstract

Background
and Purpose: The myelin oligodendrocyte glycoprotein (MOG) antibody is detected at a high rate in childhood acquired demyelinating syndrome (ADS). This study aimed to determine the diagnostic value of the MOG antibody in ADS and the spectrum of MOGantibody-positive demyelinating diseases in children.
Methods
This study included 128 patients diagnosed with ADS (n=94) or unexplained encephalitis (n=34). The MOG antibody in serum was tested using an in-house live-cell-based immunofluorescence assay.
Results
The MOG antibody was detected in 48 patients (46 ADS patients and 2 encephalitis patients, comprising 23 males and 25 females). Acute disseminated encephalomyelitis (ADEM) (35.4%) was the most-common diagnosis, followed by the unclassified form (17.4%), isolated optic neuritis (ON) (15.2%), neuromyelitis optica spectrum disorder (13.0%), multiple sclerosis (MS) (10.8%), other clinically isolated syndromes [monophasic event except ADEM, isolated ON, or transverse myelitis (TM)] (8.7%), and unexplained encephalitis (4.3%). At the initial presentation, 35 out of the 46 patients with ADS had brain lesions detected in magnetic resonance imaging, and 54% of these 35 patients had encephalopathy. Nine of the 11 patients without brain lesions exhibited only ON. Thirty-nine percent of the patients experienced a multiphasic event during the mean follow-up period of 34.9 months (range 1.4–169.0 months). Encephalopathy at the initial presentation was frequently confirmed in the monophasic group (p= 0.011).
Conclusions
MOG antibodies were identified in all pediatric ADS phenotypes except for monophasic TM. Therefore, the MOG antibody test is recommended for all pediatric patients with ADS, especially before a diagnosis of MS and for patients without a clear diagnosis.

Keyword

demyelinating disease; myelin oligodendrocyte glycoprotein; antibodies
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